Abstract

Background: Epidermolysis Bullosa (EB) is a rare genetic disease characterized by the continuous and chronic onset of bullous lesions on the skin and mucous membranes, which appear both spontaneously and due to minimal mechanical traction. Based on the skin layer in which these lesions are located, four subtypes are identified and the severity varies according to them.
Objective: As newborns with EB have to be handled with extreme care and delicacy, this work aims to report the management of a newborn with EB and to identify useful information in the literature for optimal care for these infants, focusing primarily on nursing care.
Methods: The medical-nursing assistance to a newborn with Junctional EB hospitalized at the Subintensive of Infancy Unit of Regina Margherita Children’s Hospital is reported; a literature search wasconducted to look for evidence on the nursing management of these patients.
Results: Patients with EB require complex and continuous medical-nursing management, aimed at avoiding rubbing that causes or worsen skin lesions, reducing acute and chronic pain related to skin lesions, preventing infections associated with lesions, and possible malabsorption when the gastrintestinal tract is involved.
Conclusion: This paper focuses on the management of EB, a rare, but extremely serious disease, with enormous care complexity. It reveals the huge importance of the medical-nursing collaboration in the patient care, with regards to pain and skin management, wound dressing and associated complications, but also necessary devices.